PRIMARY PULMONARY HYPERTENSION IN EIGHT PATIENTS INCLUDING A MOTHER AND HER DAUGHTER

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Abstract
Summary: Primary or idiopathic pulmonary hypertension was until recently considered a rare disorder. Since its widespread recognition in 1957 it has become evident that, while it is uncommon, it is far from rare. The present report of eight cases seen in one year substantiates this.The clinical picture is fairly characteristic and is described in this paper. Study of the jugular venous pulse would avoid many of the errors and delays in diagnosis. The course of the disease is one of progression at a variable rate, leading to death in right heart failure or from sudden arrhythmia. In this series the progression was rapid and often of a malignant nature.Cardiac catheterization will confirm the diagnosis and assess the pulmonary vascular resistance. The effects of drugs on this resistance can also be studied. This is of particular importance, as there is at present no effective treatment for this disease.Pulmonary function studies show no evidence of major disorder in the lungs. Polycythæmia and arterial desaturation are common. These are not always associated with a right‐to‐left shunt through a patent foramen ovale.The increasing frequency of reports of primary pulmonary hypertension occurring in relatives suggests that congenital or geographic factors may be of importance in the pathogenesis.
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